China Daily Global Edition (USA)
Hawking puts ALS patients in spotlight
The recent death of Stephen Hawking from amyotrophic lateral sclerosis had great resonance in China. The British physicist was an icon in the country, not just because of his intellect and the visits he made, but also because he was an inspirational figure for Chinese people with ALS, a fatal illness.
Despite being given just a couple of years to live after being diagnosed at age 21, Hawking lived for another 55 years — becoming the world’s longest-surviving ALS patient — married twice and had three children. While his body was in a wheelchair, his mind travelled the universe.
Hawking inspired people across the world thanks to his contribution to science and “his courage and persistence with his brilliance and humour”, according to a statement issued by his children.
During a visit to China in 2006, Hawking made a point of speaking publicly on June 21 — on which Global ALS Day is marked every year — telling Chinese patients, “Physical disability should not stop you from moving forward, just as I do.”
Cui Liying, director of the department of neurology at Peking Union Medical College Hospital, said an estimated 100,000 people in China have ALS, which is known in the West as Lou Gehrig’s disease after a well-known baseball player who had the illness.
Although the average life expectancy of Chinese patients is three and a half years, about half of them die within 30 months, while 20 percent live about five years, and only 10 percent survive for more than 10 years, Cui said.
She added that global research into the illness has progressed slowly, and researchers have not yet been able to determine the cause or identify a cure.
“Although ALS is not yet curable, that doesn’t mean it cannot be treated. Treatment refers to not just extending people’s life spans by the use of drugs, but also careful nursing, which is important to reduce pain and maintain a good quality of life,” she said.
“We have found that the average life span of patients in China is rising as a result of better medical treatment and nutrition, but we haven’t done a thorough survey to prove it.”
Though patients’ living standards have risen during the past decade thanks to better public awareness and improved medical care and insurance, it is still a long way from providing quality of life and dignity.
Degeneration
“In China, ALS is known as jiandong, or ‘gradual freezing’, which is an apt description of how the disease develops,” Cui said. “The patient’s muscles gradually become weak, limiting their activity as though they are freezing.”
Medically, it is the progressive degeneration of the motor neurons that eventually leads to death, she said.
The technical name provides a precise explanation of the illness. In “amyotrophic”, the “a” refers to “no”, “myo” means muscle, and “trophic” is “nourishment”. When muscles lack nourishment, they atrophy or waste away.
“Lateral” identifies the part of the spinal cord where the nerve cells that send signals to the muscles are located. As this area degenerates, it leads to “sclerosis”, or hardening.
Clinically, ALS usually starts with muscle atrophy in the hands that gradually develops into limb weakness, and eventually patients lose the ability to speak, eat, move or breathe, Cui said.
In theory, patients with advanced ALS can survive by constant use of a respirator, but that can result in complications, including lung infections and blockage of the airways by sputum or even saliva.
“The cruelest part is that the patient’s feelings, emotions, and cognitive functions are all normal, and they have to watch themselves dying step by step. They can’t scratch a place that itches or cry out when they are in pain,” Cui said.
“In China, the average onset age is about 52, so the majority of patients are middle-aged; the family breadwinner. They endure great pain inside.”
The World Health Organization lists ALS among the top five fatal illnesses, along with cancer and AIDS.
However, the illness is also designated a rare disease as a result of its low incidence, with between four to eight patients per every 100,000 people.
Cui said few patients receive a timely diagnosis because the early symptoms are not obvious and can easily be mistaken for other conditions.
“The clumsy fingers or weak limbs are ignored by many people, but the disease develops rapidly later and by then it’s too late. Early diagnosis is crucial to delaying the progress of the illness,” she said.
Meaningful existence
Shi Qingfu, who has had ALS for 14 years, is a tough, humorous man who does his best to help and encourage fellow patients.
The 35-year-old “eats” five meals a day. Two are injected directly into his stomach via a feeding tube, but he insists on chewing and swallowing the other three to stimulate his throat and stomach muscles.
After breakfast, Shi sits in front of his compute to answer questions from other patients or write about his own experiences.
His mother has to help by placing his hand on the mouse every time. He can only move one of his middle fingers, and it takes all his strength to click the mouse. Sometimes his head lolls because his neck is losing the ability to support the weight.
However, no matter how difficult his “work” becomes, Shi insists on doing it because he finds a meaningful world on the internet.
In 2005, when Shi was a junior student, he started to feel a weakness in his fingers that meant he couldn’t hold a pen. The diagnosis of ALS the following year was a bolt from the blue, given his supposedly promising future.
He overcame many difficulties to finish university, but since then he has stayed at home in Hengshui, Hebei province. Having once been desperate in the face of the fatal illness, his optimism and courageous attitude have gradually calmed him down.
His name online is Shitou, or “stone”, which is the literal meaning of his family name and also describes his steadfast personality.
His friends hail Shitou as an ALS “encyclopedia” because he tries to learn as much as possible about the illness and records the changes in his own body.
Linking theory to practice, he uses his personal experiences to help other patients.
Over the years, he has answered more than 10,000 queries from patients and their families on forums, websites and social media. He tries to encourage them with humorous remarks.
“I have made many friends through the internet. We share the same experiences, so many have been in touch. It makes me realize the value of my existence,” he said.
“Life is about constantly learning and choosing. I chose to use my knowledge to help other patients because few people travel this road and it requires courage to join in. If everyone did something, ALS patients could live better lives.”
Melting hearts
Faced with such a debilitating illness, many patients in China lack the strength to fight back, especially as until recently ALS was little known in the country.
In the West, ALS has been known for many years. In addition to Gehrig, the baseball player, the global fame of Hawking and the Ice Bucket Challenge in 2014 also helped to raise awareness globally.
The past decade has seen improvements in China, with greater social awareness and improved healthcare, according to Zhang Yanling, head of the Chinese Medical Doctor Association.
In 2005, the association established a charity program called “Melting the Frozen Heart”, which aims to improve the quality of life of ALS patients and raise awareness.
“A support system, involving people from different sectors of society, has taken shape during 13 years of effort,” Zhang said.
About 100 neurological experts from 20 hospitals are voluntarily engaged in patient consultation and educating people about the illness.
Zhang added that the association has received donations totaling more 60 million yuan ($9.5 million) from businesses such as pharmaceutical companies, respirator manufacturers and makers of eyetracking equipment that allows patients to read computer screens.
“Media exposure has also greatly raised public awareness,” he said.
“Reporters use their pens and cameras to convey patients’ needs and their touching stories,” he said.
“The most important part is the patients and their families who struggle a lot to fight this rare disease, impressing us and teaching us a lot.
Wang Zhenhua, director of the program, said ALS is becoming better known in China.
“When I searched for information about the illness on a Chinese search engine in 2005, there were fewer than 10,000 results, but now there are more than 5 million, so public awareness has certainly been raised,” he said.
“Last year, medication for ALS was included in the national medical insurance program as part of improvements in the conditions for drug use for rare diseases,” he added.
“However, more efforts still need to be made to help patients receive timely treatment, understand the disease in a scientific way and live dignified lives. We hope greater efforts will help us to melt more frozen hearts.”
ALS patient, in March, 2015. I chose to use my knowledge to help other patients because few people travel this road and it requires courage to join in. If everyone did something, ALS patients could live better lives.” Shi Qingfu, who was diagnosed with ALS 14 years ago